Abstract
Backgroundland-White-Garland syndrome (anomalous origin of the left coronary artery from the pulmonary artery) is a rare disease which may result in myocardial infarction, congestive heart failure and sometimes death during the early infantile period. Case presentation: A succesfully treated case of a 45-year-old mother of 2 children with Bland-White-Garland syndrome and concomitant severe mitral regurgitation is presented. Subsequent therapy consisted of ligation of the anomalous origin of the left coronary artery, anastomosis of the left internal mammary artery to the left anterior descending branch and mitral valve replacement. Continuous blood flow from the left coronary artery ostium during extracorporeal circulation and aorta clamping suggested systemic collateral supply. Conclusions: Recognition and diagnosis of Bland-White-Garland syndrome is important due to its potentially life-threatening complications.
Highlights
Anomalous origin of the left coronary artery arising from the pulmonary artery (Bland-White-Garland syndrome known as ALCAPA syndrome), is a rare congenital abnormality affecting 1 in 300.000 live births, accounting for 0,5% of cases of congenital heart disease [1]
Subsequent therapy consisted of ligation of the anomalous origin of the left coronary artery, anastomosis of left internal mammary artery (LIMA) to the left anterior descending (LAD) branch and mitral valve replacement with a mechanical prosthesis
White and Garland first reported the association of anomalous origin of left coronary artery from pulmonary artery with a hypertrophied left ventricle and attacks of dyspnea, pallor and profuse sweating in a boy who died at the age of 3,5 months [3]
Summary
Anomalous origin of the left coronary artery arising from the pulmonary artery (Bland-White-Garland syndrome known as ALCAPA syndrome), is a rare congenital abnormality affecting 1 in 300.000 live births, accounting for 0,5% of cases of congenital heart disease [1]. Subsequent therapy consisted of ligation of the anomalous origin of the left coronary artery, anastomosis of left internal mammary artery (LIMA) to the left anterior descending (LAD) branch and mitral valve replacement with a mechanical prosthesis. The left coronary artery and the pulmonary trunk were supplied by collaterals. The pulmonary arteriotomy revealed the ostium of left coronary artery on the right posterolateral wall of the pulmonary trunk, 0,5 cm above the posterior commissure of pulmonary valve. Patient's dyspnea had improved by one NYHA class with a great improvement in exercise tolerance
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